Prevalence and Risk Factors for Hyperacusis in Children: a Birth Cohort Study Rachel Humphriss MSc PhD CS Clinical Scientist (Audiology) University Hospitals Bristol NHS Foundation Trust Rachel.humphriss@uhbristol.nhs.uk The Team  Melanie Parker - Weston Area Health Trust  Amanda Hall - University of Bristol / University Hospitals Bristol NHS Foundation Trust  David Baguley - Cambridge University Hospitals NHS Foundation Trust / Anglia Ruskin University  Colin Steer – University of Bristol Definitions  Hyperacusis: an abnormal lowered tolerance to sound     (Baguley and Andersson, 2007) Phonophobia: the fear of sound Misophonia: negative reaction to specific types of soft sound Often confused: challenging to distinguish between a subjective increase in perceived intensity and the emotional reaction to this “Decreased Sound Tolerance” - Jastreboff and Jastreboff, 2013 Proposed mechanism  Heterogeneous condition  Increased gain of central auditory system  May be associated with reduced peripheral auditory sensitivity  Often comorbid with tinnitus: hyperacusis suggested as a precursor to tinnitus Associated conditions  Williams syndrome  High frequency HL  Recurrent OME  Acoustic reflexes deficient  Autistic Spectrum Disorder  Auditory hypervigilance?  Oversensitivity to light and touch  Migraine  Depression  Post-Traumatic Stress Disorder Previous studies of prevalence  Adults: 8 – 23%  Coelho et al (2007)  Cross-sectional study  506 children (from random selection of 700, one town in Brazil)  Age 5 – 12 years Hyperacusis  “Are you bothered by any kind of noise?” + identify ≥10/20 sounds from a list as being annoying + lowered LDL (5th percentile ≥ 1 frequency)  Prevalence: 3.2% Phonophobia  “Are you afraid of sounds?”  Prevalence: 9% Other studies  Sattar (2009)  100 children from a clinic  Hyperacusis more common in males 2:1 ratio  Baguley et al (2013)  88 young persons with tinnitus, 4 specialist European centres  39% had decreased sound tolerance Study aims  To estimate the prevalence of hyperacusis in 11 year old children using data from a prospective UK populationbased study  To identify any early life and auditory risk factors for hyperacusis ALSPAC       Avon Longitudinal Study of Parents and Children Prospective observational study www.bristol.ac.uk/alspac/ Former Avon region: Population about 1 million Urban, suburbs, rural, small towns All pregnant women in region with expected dates of delivery between April 1st 1991 and December 31st 1992 were invited to participate  Ethical approval for the study was obtained from the ALSPAC Law and Ethics Committee and the Local Research Ethics Committees. ALSPAC  Response rate approx 80%  N = 13,971 live infants at age 12 mths  Sample considered to be broadly representative of UK population (slightly higher levels of social advantage and lower levels of ethnic minorities)  See Boyd et al (2013) for details of cohort Prospective longitudinal studies: ALSPAC Selection of participants Pregnancy Measurement of SES / other exposures Age 7 TIME Hearing Age 9 Hearing Age 11 Hearing Measurement of other risk factors and confounders Hearing assessment session: Age 11  Pure Tone Audiometry (AC, BC) (BSA recommended procedure)  Tympanometry  Questions about sound sensitivity  “Do you ever experience over-sensitivity or distress to particular sounds?”  Avoidance?  Over-sensitivity to light/colours, touch, pain, smell or taste?  Ear protection?  Questions about tinnitus Non-auditory risk factors  Prenatal  Smoking in pregnancy  Early life  Gestational age (<37 weeks, ≥37 weeks)  Birth-weight (≤2500g, >2500g)  Gender  SCBU  Socioeconomic  Maternal education level (<16yrs, 16yrs, ≥18yrs)  Housing tenure (mortgaged/owned, private rented, council/HA)  Occupational social class (Registrar General’s Classification) Results: prevalence  N=7093  Study sample socially advantaged compared to remainder of cohort  261 reported oversensitivity to sound  Prevalence of hyperacusis = 3.68% [95% CI 3.25, 4.14]  Behavioural avoidance by 112 (42.9% hyperacusic children)  21 (8.0%) used ear protection Results: other sensory sensitivities Sensory sensitivity N (% children with hyperacusis) Light/colours 22 (8.4) Touch 17 (6.5) Smell 14 (5.4) Taste 10 (3.8) Pain 9 (3.4) Association with socioeconomic factors Reports of hyperacusis strongly associated with:  Higher maternal education Mat Ed N (%) hyperacusis N (%) remainder of sample Unadj OR [95% CI] P Adj OR [95% CI] P <16yrs 39 (16.3) 1381 (22.1) Ref GCSE 79 (32.9) 2217 (35.5) 1.26 [0.85, 1.86] 0.242 1.52 [0.97, 2.40] 0.066 A level+ 122 (50.8) 2644 (42.4) 1.63 [1.13, 2.35] 0.009 1.72 [1.08, 2.72] 0.020 Ref Association with child factors Reports of hyperacusis strongly associated with:  Male gender  Adj. OR for hyperacusis, if female = 0.64 [0.49, 0.85], p=0.002  Children re/admitted to hospital in first 4 weeks of life  Adj. OR for hyperacusis, if hospital admission = 1.98 [1.20, 3.25], p=0.007  No strong associations with birth-weight, gestational age, admission to SCBU Associations with PTA results Ear Frequ Mean dBHL N Mean dBHL with N ency without hyperacusis (Hz) hyperacusis [95% CI)] P value Linear regression (dBHL) [95% CI] Right Left Bone 500 4.81 [4.64, 4.98] 6766 5.45 [4.42, 6.48] 254 0.159 1000 3.94 [3.77, 4.11] 6826 4.67 [3.61, 5.72] 260 0.117 2000 3.72 [3.55, 3.89] 6823 3.56 [2.66, 4.46] 258 0.729 3000 3.54 [3.36, 3.72] 6772 3.13 [2.14, 4.12] 255 0.388 4000 3.37 [3.18, 3.56] 6818 3.44 [2.42, 4.47] 258 0.878 6000 6.38 [6.16, 6.60] 6759 6.98 [5.74, 8.21] 250 0.316 8000 7.97 [7.73, 8.20] 6789 8.26 [7.03, 9.49] 248 0.640 500 5.17 [5.00, 5.35] 6769 5.92 [4.69, 7.16] 253 0.115 1000 3.30 [3.13, 3.47] 6827 4.28 [3.08, 5.47] 257 0.038 2000 3.65 [3.47, 3.82] 6826 4.16 [2.98, 5.33] 257 0.291 3000 3.62 [3.44, 3.81] 6774 4.46 [3.14, 5.78] 253 0.093 4000 4.04 [3.85, 4.24] 6816 4.90 [3.58, 6.21] 256 0.110 6000 7.40 [7.17, 7.63] 6765 8.15 [6.71, 9.59] 249 0.238 8000 7.57 [7.33, 7.81] 6795 9.06 [7.57, 10.56] 247 0.024 500 -0.167 [-0.32, -0.01] 6796 0.94 [0.04, 1.84] 249 0.008 1000 -1.19 [-1.34, -1.04] 6800 -0.79 [-1.64, 0.06] 253 0.315 2000 1.91 [1.74, 2.08] 6795 1.85 [0.91, 2.78] 251 0.893 Strong associations inconsistent and diminished when adjusted for covariates Differences only approx. 1dBHL: not clinically significant Associations with other audiological outcomes Tinnitus  109/260 (41.9%) children reporting hyperacusis also reported tinnitus  Chi2=25.3, p<0.0001 Tympanometry  Children were not more likely to have OME (at age 11) Conclusions: prevalence  ALSPAC is the largest population-based study to look at hyperacusis in children  More generalisable than Coelho et al (2007)  Prevalence of hyperacusis in 11 year old children is estimated at 3.68% [3.25, 4.14]  Approx. one child per class affected     42.9% of these children showed avoidance behaviours Only 8% used ear protection A small proportion were sensitive to light / colours More common in boys Conclusions: SES  Associated with social advantage as measured by maternal education  Child more articulate?  Greater awareness of health issues?  Parents more attentive to health needs?  Fewer “life issues” to worry about?  Unusual as most health conditions are associated with social disadvantage  Socially advantaged sample so may have overestimated prevalence Conclusions: neonatal health  Hyperacusis more prevalent in children admitted to hospital in first month of life Causal:  Adverse effect on auditory neurodevelopment?  Adverse effect on brain development which predisposes child to later behavioural / emotional problems? Non-causal:  Parents of child with difficult start in life might be hyper-vigilant about child’s health? Conclusions: auditory outcomes  Strong association with tinnitus  No consistent associations with hearing thresholds  No associations with middle ear function (at age 11)  Middle ear history not examined – early OME may be a risk factor  LDLs not measured: have low sensitivity / specificity for hyperacusis Acknowledgements  We are extremely grateful to all the families who took part in this study, the midwives for their help in recruiting them, and the whole ALSPAC team which includes interviewers, computer and laboratory technicians, clerical workers, research scientists, volunteers, managers, receptionists and nurses.  The UK Medical Research Council (Grant ref: 74882), the Wellcome Trust (Grant ref: 076467) and the University of Bristol provide core support for ALSPAC References  Baguley DM, Bartnik G, Kleinjung T, Savastano M, Hough EA. Troublesome tinnitus in childhood and adolescence: data from expert centres. Int J Ped Otolaryngol 2013. 77(2): 248-251.  Boyd R, Golding J, Macleod J, et al. Cohort profile: the ‘Children of the 90s’; the index offspring of the Avon Longitudinal Study of Parents and Children. Int J Epidemiol 2013; 42: 111-127.  Coelho CB, Sanchez TG, Tyler RS. Hyperacusis, sound annoyance, and loudness hypersensitivity in children. Progress in Brain Research 2007; 166: Chap 15: 169-178.  Sattar N. A study of hyperacusis in 100 normally-hearing children. Arch Dis Child 2009. 84(Suppl 1): A98 (G251).